Myocardial disturbances of intermediary metabolism in Barth syndrome

被引：4

作者：

Greenwell, Amanda A. ^{[1
,2
]}

Dakhili, Seyed Amirhossein Tabatabaei ^{[1
,2
]}

Ussher, John R. ^{[1
,2
]}

机构：

[1] Univ Alberta, Fac Pharm & Pharmaceut Sci, Edmonton, AB, Canada

[2] Univ Alberta, Women & Childrens Hlth Res Inst, Edmonton, AB, Canada

来源：

FRONTIERS IN CARDIOVASCULAR MEDICINE | 2022年 / 9卷

基金：

加拿大健康研究院;

关键词：

Barth syndrome (BTHS); cardiomyopathy; cardiac energetics; glucose oxidation; fatty acid oxidation; ketone oxidation; HEART-FAILURE; FAILING HEART; KETONE-BODIES; CARDIOMYOPATHY; CARDIOLIPIN; MECHANISMS; REPERFUSION; DEFICIENCY; SUBSTRATE; OXIDATION;

D O I：

10.3389/fcvm.2022.981972

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Barth Syndrome (BTHS) is a rare X-linked mitochondrial disorder due to mutations in the gene TAFAZZIN, which leads to immature cardiolipin (CL) remodeling and is characterized by the development of cardiomyopathy. The immature CL remodeling in BTHS results in electron transport chain respiratory defects and destabilization of supercomplexes, thereby impairing ATP production. Thus, BTHS-related cardiomyopathy appears to share metabolic characteristics of the failing heart being an "engine out of fuel." As CL associates with numerous mitochondrial enzymes involved in ATP production, BTHS is also characterized by several defects in intermediary energy metabolism. Herein we will describe the primary disturbances in intermediary energy metabolism relating to the heart's major fuel sources, fatty acids, carbohydrates, ketones, and amino acids. In addition, we will interrogate whether these disturbances represent potential metabolic targets for alleviating BTHS-related cardiomyopathy.

引用

页数：8

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